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Body composition in children with galactosaemia
B Panis1, P Ph Forget, F H Nieman
1Dept. of Paediatrics and Laboratory of Inherited Metabolic Diseases, University Hospital Maastricht, Maastricht, The Netherlands.
Journal of Inherited Metabolic Disease
|January 26, 2006
Summary
Children with classical galactosaemia have abnormal body composition and decreased height. Insulin-like growth factor-I (IGF-I) levels and soy intake may influence these findings in patients with galactose-1-phosphate uridyltransferase (GALT) deficiency.
Area of Science:
- Biochemistry
- Pediatrics
- Human Genetics
Background:
- Classical galactosaemia is an inherited metabolic disorder caused by galactose-1-phosphate uridyltransferase (GALT) deficiency.
- Patients may experience abnormal body composition due to disease-related factors and diet.
- Previous studies have not focused on body composition in this population.
Purpose of the Study:
- To evaluate the body composition of children diagnosed with classical galactosaemia.
- To investigate potential correlations between body composition, growth parameters, and nutritional intake.
Main Methods:
- Dual-energy X-ray absorptiometry (DXA) was used to assess body composition (fat mass and lean tissue mass).
- Measurements were adjusted for height, and compared to mid-parental target height Z-scores.
- Regression analysis explored relationships between body composition, insulin-like growth factor-I (IGF-I) Z-scores, and dietary intake (including soy).
Main Results:
- Patients exhibited decreased height Z-scores compared to their mid-parental target height.
- Adjusted fat mass (FM) and lean tissue mass (LTM) were found to be decreased.
- LTM and height Z-scores correlated with IGF-I Z-scores, while FM correlated with soy intake.
Conclusions:
- Children with classical galactosaemia present with reduced height and altered body composition.
- Decreased IGF-I levels and/or soy intake may contribute to these observed abnormalities.
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